Rev Esp Med Nucl Imagen Mol. 2014;33(2):99–102 Clinical note Autoimmune lymphoproliferative syndrome and non-Hodgkin lymphoma: What 18 F-fluorodeoxyglucose positron emission tomography/computed tomography can do in the management of these patients? Suggestions from a case report A. Cistaro a,∗,1 , F. Pazè b , S. Durando b , M. Cogoni c , R. Faletti d , S. Vesco e , S. Vallero f , N. Quartuccio g , G. Treglia h , U. Ramenghi f a Positron Emission Tomography Centre, IRMET S.p.A., Euromedic Inc., Turin, Italy b Department of Radiology, San Luigi Gonzaga Hospital, Turin, Italy c Department of Neuroradiology, CTO Hospital, Turin, Italy d Department of Radiology, San Giovanni Battista Hospital, Turin, Italy e Department of Physical Medicine and Rehabilitation, Martini Hospital, Turin, Italy f Pediatric Department, University of Turin, Turin, Italy g Department of Radiological Sciences, Nuclear Medicine Unit, University of Messina, Messina, Italy h Department of Nuclear Medicine and PET/CT Centre, Oncology Institute of Southern Switzerland, Bellinzona, Switzerland a r t i c l e i n f o Keywords: Fas gene PET Non-Hodgkin lymphoma Rituximab Autoimmune lymphoproliferative syndrome a b s t r a c t A young patient with undefined autoimmune lymphoproliferative syndrome (ALPS-U) and low back pain underwent a CT and MRI study that showed enhancing vertebral lesions, some pulmonary nodules and diffuse latero-cervical lymphadenopathy. A 18 F-FDG-PET/CT scan showed many areas of intense 18 F-FDG uptake in multiple vertebrae, in some ribs, in the sacrum, in the liver, in both lungs, in multiple lymph nodes spread in the cervical, thoracic and abdominal chains. A bone marrow biopsy showed a “lym- phomatoid granulomatosis”, a rare variant of B-cell non-Hodgkin lymphoma (NHL). After the treatment, the 18 F-FDG-PET/CT scan showed a complete metabolic response. © 2013 Elsevier España, S.L. and SEMNIM. All rights reserved. Síndrome linfoproliferativo autoinmune indefinido y linfoma no-Hodgkin. ¿Qué puede aportar la exploración 18 F-FDG-PET/CT al tratamiento de estos pacientes? Sugerencias a partir de un caso Palabras clave: gen Fas PET Linfoma no Hodgkin Rituximab Síndrome linfoproliferativo autoinmune r e s u m e n Un paciente joven con síndrome linfoproliferativo autoinmune indefinido (ALPS-U) y dolor lumbar se sometió a una tomografía computarizada y a una resonancia magnética, estudios que mostraron varias lesiones vertebrales, algunos nódulos pulmonares y adenopatías laterocervicales difusas. Una exploración 18 F-FDG-PET/TC reveló áreas de captación intensa de 18 F-FDG en múltiples vértebras, algunas costillas, sacro, hígado, ambos pulmones y en varios ganglios linfáticos repartidos en las cadenas cervicales, torácica y abdominal. La biopsia de médula ósea diagnosticó una “granulomatosis linfomatoide”, una variante poco frecuente de Linfoma no-Hodgkin de células B (LNH). Tras el tratamiento, la exploración 18 F-FDG-PET/TC demostró una respuesta metabólica completa. © 2013 Elsevier España, S.L. y SEMNIM. Todos los derechos reservados. Introduction The autoimmune lymphoproliferative syndrome (ALPS) is a rare genetic disorder involving the Fas apoptotic pathway in lympho- cytes. Fas (also called Apo-1 and CD95) is a death receptor belonging to the tumor necrosis factor receptor (TNFR) super-family that induces cell death, when triggered by Fas ligand (FasL or CD95L). 1 ∗ Corresponding author. E-mail address: a.cistaro@irmet.com (A. Cistaro). 1 Coordinator of Italian PET-Pediatric Study Group. Fas pathway maintains the lymphocyte homeostasis and is involved in switching-off the immune response, limiting clonal expansion of lymphocytes, favoring peripheral toler- ance. Fas is highly expressed by cytotoxic T cells and NK cells and plays an important role in preventing cancer. 2 Thus, patients with Fas mutations show chronic lymphoproliferation, hepatosplenomegaly and even autoimmune manifestations. Fur- thermore subjects with ALPS have propensity to develop Hodgkin and non-Hodgkin lymphoma with a risk of 51 and 14 times greater than expected, respectively. 3,4 We report a case of a young patient affected by ALPS, who developed a “lymphomatoid granulomatosis”, a rare variant of B-cell non-Hodgkin lymphoma. In this case fluorine-18-fluorodeoxyglucose positron emission 2253-654X/$ – see front matter © 2013 Elsevier España, S.L. and SEMNIM. All rights reserved. 2253-8089/$ Gen